Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy

Introduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demy...

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Main Authors: D. A. Grishina, N. A. Suponeva, A. S. Rizvanova
Format: Article
Language:Russian
Published: ABV-press 2020-08-01
Series:Нервно-мышечные болезни
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Online Access:https://nmb.abvpress.ru/jour/article/view/383
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author D. A. Grishina
N. A. Suponeva
A. S. Rizvanova
author_facet D. A. Grishina
N. A. Suponeva
A. S. Rizvanova
author_sort D. A. Grishina
collection DOAJ
description Introduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demyelinating polyneuropathy, characterized by benign disease course, minimal motor involvement and not required pathogenic therapy.Materials and methods. 8 patients (7 men (87 %) and 1 woman (13 %) at the age of 52–77 years) with atypical variants of chronic inflammatory demyelinating polyneuropathy were analyzed: 5 patients (62.5 %) with asymmetric variant – multifocal acquired demyelinating sensorimotor neuropathy and 3 patients (37.5 %) with sensory variant. All patients were observed at the Research Center of Neurology for the period of 2016– 2019. In each patient the proper clinical and laboratory evaluation was performed along with nerve conduction study and nerve ultrasound.Results. The disease duration at the time of first visit was 1–8 years. By INCAT disability score 3 (37.5 %) patients had 0 points (normal), 3 (37.5 %) patients – 1 point and 1 patient had 2 (25 %) points. Nerve conduction study showed multifocal, asymmetric demyelinating changes in motor nerves. For the whole period of observation all patients were stable, so no one required pathogenic therapy.Conclusion. Chronic inflammatory demyelinating polyneuropathy is a clinically heterogeneous disorder, required clinical suspicion in all patients over 50 years with features of multiple nerve involvement; nerve conduction study helps to detect typical changes, including subclinical ones. The primary strategy of management typical and atypical disease variants in stable course and minimal symptoms is a case follow-up with precise assessment of advantages and disadvantages of pathogenic therapy.
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spelling doaj-art-889fc694b75d4d06929cf467af35e5152025-08-20T03:37:12ZrusABV-pressНервно-мышечные болезни2222-87212413-04432020-08-01102223010.17650/2222-8721-2020-10-2-22-30268Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapyD. A. Grishina0N. A. Suponeva1A. S. Rizvanova2The Research Center of NeurologyThe Research Center of NeurologyThe Research Center of NeurologyIntroduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demyelinating polyneuropathy, characterized by benign disease course, minimal motor involvement and not required pathogenic therapy.Materials and methods. 8 patients (7 men (87 %) and 1 woman (13 %) at the age of 52–77 years) with atypical variants of chronic inflammatory demyelinating polyneuropathy were analyzed: 5 patients (62.5 %) with asymmetric variant – multifocal acquired demyelinating sensorimotor neuropathy and 3 patients (37.5 %) with sensory variant. All patients were observed at the Research Center of Neurology for the period of 2016– 2019. In each patient the proper clinical and laboratory evaluation was performed along with nerve conduction study and nerve ultrasound.Results. The disease duration at the time of first visit was 1–8 years. By INCAT disability score 3 (37.5 %) patients had 0 points (normal), 3 (37.5 %) patients – 1 point and 1 patient had 2 (25 %) points. Nerve conduction study showed multifocal, asymmetric demyelinating changes in motor nerves. For the whole period of observation all patients were stable, so no one required pathogenic therapy.Conclusion. Chronic inflammatory demyelinating polyneuropathy is a clinically heterogeneous disorder, required clinical suspicion in all patients over 50 years with features of multiple nerve involvement; nerve conduction study helps to detect typical changes, including subclinical ones. The primary strategy of management typical and atypical disease variants in stable course and minimal symptoms is a case follow-up with precise assessment of advantages and disadvantages of pathogenic therapy.https://nmb.abvpress.ru/jour/article/view/383chronic inflammatory demyelinating polyneuropathyatypical variantmultifocal acquired demyelinating sensorimotor neuropathylewis–sumner syndromesensory variantnerve conduction study
spellingShingle D. A. Grishina
N. A. Suponeva
A. S. Rizvanova
Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
Нервно-мышечные болезни
chronic inflammatory demyelinating polyneuropathy
atypical variant
multifocal acquired demyelinating sensorimotor neuropathy
lewis–sumner syndrome
sensory variant
nerve conduction study
title Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
title_full Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
title_fullStr Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
title_full_unstemmed Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
title_short Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy
title_sort atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course a clinical observation for 8 patients without pathogenic therapy
topic chronic inflammatory demyelinating polyneuropathy
atypical variant
multifocal acquired demyelinating sensorimotor neuropathy
lewis–sumner syndrome
sensory variant
nerve conduction study
url https://nmb.abvpress.ru/jour/article/view/383
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AT nasuponeva atypicalvariantsofchronicinflammatorydemyelinatingpolyneuropathywithbenigncourseaclinicalobservationfor8patientswithoutpathogenictherapy
AT asrizvanova atypicalvariantsofchronicinflammatorydemyelinatingpolyneuropathywithbenigncourseaclinicalobservationfor8patientswithoutpathogenictherapy